Memory loss and frontal cognitive dysfunction in a patient with adult-onset neuronal intranuclear inclusion disease

Kunihiko Araki, Jun Sone, Yusuke Fujioka, Michihito Masuda, Reiko Ohdake, Yasuhiro Tanaka, Tomohiko Nakamura, Hirohisa Watanabe, Gen Sobue

Research output: Contribution to journalArticlepeer-review

14 Citations (Scopus)

Abstract

Neuronal intranuclear inclusion disease (NIID) is an uncommon progressive neurodegenerative disorder. Adult-onset NIID can result in prominent dementia. We herein describe the case of a 74-year-old man who presented with dementia, cerebellar ataxia, neuropathy, and autonomic dysfunction. Diffusion-weighted imaging showed hyperintensity of the corticomedullary junction. Fluid-attenuated inversion recovery images showed frontal-dominant white matter hyperintensity. NIID was diagnosed from the presence of intranuclear inclusions in a skin biopsy sample. Neuropsychological testing revealed memory loss and frontal cognitive dysfunction, especially in relation to language and executive functions. We were therefore able to confirm the association of NIID with cognitive dysfunction.

Original languageEnglish
Pages (from-to)2281-2284
Number of pages4
JournalInternal Medicine
Volume55
Issue number16
DOIs
Publication statusPublished - 2016
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Internal Medicine

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